Initial Presentation of Tuberous Sclerosis Complex in an Adult Woman with Hemorrhage from Bilateral, Giant, Multi-Focal Renal Angiomyolipomas

Carrie Stewart, Ashley Richman, Jacob Scheibal and Jonathan Silberstein

Department of Urology, Tulane University, USA
Department of Urology, The University of Queensland School of Medicine, USA
Department of Radiology, Ochsner Medical Center, USA

^*Correspondance to: Carrie Stewart 

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Abstract:

Renal angiomyolipomas (AML’s) are benign tumors containing smooth muscle, adipose, and aberrant vasculature and can cause hypertension, renal failure and life threatening hemorrhage. In Tuberous sclerosis complex (TSC), AMLs are often bilateral and can be too numerous to count, up to 89% and 76% respectively. The disease is autosomal dominant and affects multiple anatomic systems. No single feature is diagnostic for TSC, making complete evaluation for major and minor signs requisite. We report a case of a hemorrhagic AML in a woman with giant, bilateral, multifocal AML and hypovolemic shock requiring emergent endovascular treatment of unilateral AML hemorrhage.

Keywords:

Angiomyolipoma; Sirolimus; mTOR; Giant angiomyolipoma

Cite the Article:

Stewart C, Richman A, Scheibal J, Silberstein J. Initial Presentation of Tuberous Sclerosis Complex in an Adult Woman with Hemorrhage from Bilateral, Giant, Multi-Focal Renal Angiomyolipomas. Ann Clin Case Rep. 2016; 1: 1147.